title: Cholestatic disorders of infancy--aetiology and outcome.
abstract: The relative frequency of causes of cholestatic disorders of infancy in a developing area was established in a prospective study. During a 10-year period, 145 infants with conjugated hyperbilirubinaemia were investigated. Intrahepatic disorders accounted for 68 per cent with no identifiable cause (idiopathic hepatitis) in the majority. Syphilis, urinary tract infection and septicaemia together made up 30 per cent of intrahepatic causes with metabolic disorders accounting for 12 per cent. Outcome in those with idiopathic hepatitis, and those treated for syphilis and UTI was relatively good. Complete recovery from syphilitic hepatitis on average took 11 months. Extrahepatic disorders occurred in 32 per cent and were almost entirely due to biliary atresia. Results of hepatic portoenterostomy for biliary atresia were poor because of late referral in many instances. Compared to developed countries, infantile cholestasis in developing areas is more commonly associated with treatable bacterial infection. Referring agencies should be aware of this fact and the need for early referral of cases with possible biliary atresia.
authors.affiliation: Institute of Child Health, University of Cape Town, Republic of South Africa.
authors.forename: C
authors.initials: C
authors.lastname: Motala
authors.forename: J D
authors.initials: JD
authors.lastname: Ireland
authors.forename: I D
authors.initials: ID
authors.lastname: Hill
authors.forename: M D
authors.initials: MD
authors.lastname: Bowie
mesh_terms: D002779:Cholestasis / Q000209:etiology* / Q000628:therapy; D005260:Female; D006801:Humans; D006932:Hyperbilirubinemia / Q000209:etiology; D007223:Infant; D008297:Male; D011446:Prospective Studies; D013019:South Africa